Indian Journal of Public Health Research & Development

Open Access Open Access  Restricted Access Subscription Access
Open Access Open Access Open Access  Restricted Access Restricted Access Subscription Access

Fetal Hemoglobin & Liver Dysfunction in Sickle Cell Crisis


Affiliations
1 Department of Pediatrics, Pt JNM Medical College, Raipur, CG, India
2 Department of Biochemistry, Pt JNM Medical College, Raipur, CG, India
3 Department of Biochemistry, Saraswati Institute of Medical College, Hapur, UP, India
     

   Subscribe/Renew Journal


Fetal hemoglobin is one of the major factors that alters the clinical course of disease. A study of 45 patients of sickle cell crisis was carried out in Department of Biochemistry&Department of Pediatrics, Pt JNM Medical College, Raipur, Chhattisgarh between June 2007 to July 2009. All patients had homozygous sickle cell anemia admitted in our institutional hospital for sickle cell crisis. Patients were diagnosed by cellulose acetate electrophoresis and adult, fetal and sickle hemoglobin was quantitated by cation exchange HPLC (Biorad Variant hemoglobin testing system). The patients were divided into three groups based on the Hb F concentration in whole blood. Group I, II and III had 20 percent HbF respectively. HbF is higher in lesser age group but it was not statistically significant. The average Sickle cell crisis per year and recurrent events were 2.7, 3.2 and 1.4 in group-I, group-II and group-III respectively. So in group-III the crisis/year was less than compared to other two groups. The average number of blood transfusions in groups I, II&III till the study period was 14, 7.1 and 3.1 respectively. So there was a downward trend of no. of B.T with increasing level of HbF. There was significant drop in the requirement of B.T , reccurence of sickle cell crisis in the patients above HbF level of 20%. The mean size in centimeters of spleen was lower with increasing HbF level. but there was no significant difference in liver size. SGOT&SGPT was in normal reference range in patients with HbF level > 20% whereas it was abnormal in patients with HbF levels < 20%.. There was no significant difference in hematological parameters like Hb, MCV, HCT, RBC, MCH&MCHC between any of the groups. However platelet count was elevated with HbF levels < 20% and in normal range with HbF levels > 20%.
Subscription Login to verify subscription
User
Notifications
Font Size


  • Alabulaali MK. Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes. Annals of Thoracic Medicine 2007;Vol 2 Issue 4:158- 162.

  • Bailey K, Morris JS, Thomas P & Serjeant GR. Fetal hemoglobin and early manifestations of homozygous sickle cell disease. Archives of Diseases in Childhood 1992;67:517-520.

  • Baum KF, Dunn DT, Maude GH & Sergeant GR. The painful crisis of homozygous sickle cell disease: a study of risk factors. Archives of internal medicine 1987;147:1231-1234

  • Bertles JF, Rabinowitz JFR & Dobler J. Hemoglobin interaction: modification of solid phase composition in the sickling phenomenon. Science 1970;169:375- 377.

  • Bertles JF & Milner PF. Irreversibly sickled erythrocytes: a consequence of hetergenous distribution of hemoglobin types in sickle cell anemia. Journal of Clinical Investigation 1992;47:1731.

  • Bhatia HM, Rao VR. Genetic atlas of the Indian tribes. Published in 1986 by Institute of immunohaematology, ICMR, Bombay, India.

  • Darleen R Powars, Joyce N Weiss, Linda S Chan & WA Schroeder. Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia ?. Blood 1984;Vol. 63 No. 4: 921-926.

  • Donaldson A, Thomas P, Serjeant BE, Serjeant GR. Fetal hemoglobin in homozygous sickle cell disease: a study of patients with low HBF levels. Clin Lab Haem 2001;23:285-289.

  • Jackson JF, Odom JL & Bell WN. Amelioration of sickle cell disease by persistent fetal hemoglobin. Journal of American Medical Association 1961;177:867-869.

  • Powars DR, Schroeder WA, Weiss JN. Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia. J Clin. Invest. 1980;65:732-740.

  • Kadam MD, Mukherjee MB, Colah RB, Gangakhedkar RR, Mohanty D. Is fetal hemoglobin level a prognostic indicator for severity of sickle cell disease?. Ind. J Hum. Genet. 1996;2:43-49.

  • Kar BC, Satapathy RK, Kulozik AE et al. Sickle cell disease in state of Orrisa state, India. The Lancet 1986:Nov. 22:1198-1201.

  • Perrine RP, Pembrey ME, John P, Perrine S & Shoup F. Natural history of sickle cell anemia in Saudi Arabia. Annals of internal Medicine 1978;88:1-6.

  • Richard S, Billet HH. Liver function tests in sickle cell disease. Clin. Lab. Haem. 2002;24:21-27.

  • Serjeant GR. Irreversibly sickled cells and splenomegaly in sickle cell anemia. British Journal of Hematology 1970;19:635-641.

  • Sergeant GR. Fetal hemoglobin in homozygous sickle cell disease. Clinical hematology 1975;4:109-122.


Abstract Views: 286

PDF Views: 0




  • Fetal Hemoglobin & Liver Dysfunction in Sickle Cell Crisis

Abstract Views: 286  |  PDF Views: 0

Authors

Sumanta Panigrahi
Department of Pediatrics, Pt JNM Medical College, Raipur, CG, India
Praveen Sablania
Department of Biochemistry, Pt JNM Medical College, Raipur, CG, India
P. K. Khodiar
Department of Biochemistry, Pt JNM Medical College, Raipur, CG, India
J. R. Keshari
Department of Biochemistry, Saraswati Institute of Medical College, Hapur, UP, India
Pradeep Kumar Patra
Department of Biochemistry, Pt JNM Medical College, Raipur, CG, India

Abstract


Fetal hemoglobin is one of the major factors that alters the clinical course of disease. A study of 45 patients of sickle cell crisis was carried out in Department of Biochemistry&Department of Pediatrics, Pt JNM Medical College, Raipur, Chhattisgarh between June 2007 to July 2009. All patients had homozygous sickle cell anemia admitted in our institutional hospital for sickle cell crisis. Patients were diagnosed by cellulose acetate electrophoresis and adult, fetal and sickle hemoglobin was quantitated by cation exchange HPLC (Biorad Variant hemoglobin testing system). The patients were divided into three groups based on the Hb F concentration in whole blood. Group I, II and III had 20 percent HbF respectively. HbF is higher in lesser age group but it was not statistically significant. The average Sickle cell crisis per year and recurrent events were 2.7, 3.2 and 1.4 in group-I, group-II and group-III respectively. So in group-III the crisis/year was less than compared to other two groups. The average number of blood transfusions in groups I, II&III till the study period was 14, 7.1 and 3.1 respectively. So there was a downward trend of no. of B.T with increasing level of HbF. There was significant drop in the requirement of B.T , reccurence of sickle cell crisis in the patients above HbF level of 20%. The mean size in centimeters of spleen was lower with increasing HbF level. but there was no significant difference in liver size. SGOT&SGPT was in normal reference range in patients with HbF level > 20% whereas it was abnormal in patients with HbF levels < 20%.. There was no significant difference in hematological parameters like Hb, MCV, HCT, RBC, MCH&MCHC between any of the groups. However platelet count was elevated with HbF levels < 20% and in normal range with HbF levels > 20%.

References